NEK1 (NIMA-Related Kinase 1) is a serine/threonine-protein kinase that plays a multifaceted and critical role in cellular homeostasis. It is widely expressed and involved in key biological processes, most notably the DNA Damage Response (DDR) and cilium assembly and function. NEK1 kinase activity is regulated in response to genotoxic stress, where it facilitates the repair of double-strand breaks alongside proteins like ATM and ATR. Simultaneously, it localizes to the basal body of the primary cilium, governing ciliary structure and trafficking. The paramount importance of NEK1 is highlighted by its strong genetic association with Amyotrophic Lateral Sclerosis (ALS); loss-of-function mutations in NEK1 are recognized as one of the most common genetic causes of familial and sporadic ALS. Furthermore, biallelic mutations in NEK1 are responsible for the severe ciliopathy known as short-rib thoracic dysplasia, underscoring its vital role in skeletal development. Thus, NEK1 represents a crucial molecule at the crossroads of genome integrity, ciliary function, and human neurodegenerative and developmental diseases.